Yalin Tolga Yaylali1, Samet Yilmaz2, Gulsum Akgun-Cagliyan3, Oguz Kilic2, Emrah Kaya4, Hande Senol5, Furkan Ozen2. 1. Department of Cardiology, Faculty of Medicine, Pamukkale University, Denizli, Turkey, yaylalimd@gmail.com. 2. Department of Cardiology, Faculty of Medicine, Pamukkale University, Denizli, Turkey. 3. Department of Hematology, Faculty of Medicine, Pamukkale University, Denizli, Turkey. 4. Division of Cardiology, Ardahan State Hospital, Ardahan, Turkey. 5. Department of Biostatistics, Faculty of Medicine, Pamukkale University, Denizli, Turkey.
Abstract
BACKGROUND: Pulmonary hypertension (PH) can complicate the course of myeloproliferative neoplasms (MPNs). Echocardiography is a useful noninvasive screening test for PH in populations at risk. We aimed to investigate the echocardiographic evidence of PH and clinical characteristics of patients with MPNs. METHODS: This study included 197 patients with MPNs (mean age, 59 ± 14 years; females, 53%; mean disease duration, 3.4 ± 2.8 years). Clinical and laboratory characteristics, including JAK2V617F mutation status, were obtained. All participants underwent a comprehensive transthoracic echocardiographic examination. The echocardiographic evidence of PH was defined as systolic pulmonary artery pressure (SPAP) ≥40 mm Hg. RESULTS: Overall, 11 patients (5.5%) with SPAP ≥40 mm Hg had echocardiographic evidence of PH. Patients with myelofibrosis had echocardiographic evidence of PH more often than patients with other MPNs (p < 0.001). Disease duration since the diagnosis of MPNs was 6.7 ± 4.6 years in the PH group and 3.1 ± 2.5 years in the non-PH group (p < 0.001). There was a weak positive correlation between SPAP values and time since diagnosis (r = 0.236, p =0.001). JAK2V617F mutation was not associated with PH. In multivariate logistic regression analysis, the presence of myelofibrosis (odds ratio [OR]: 22.177, 95% CI: 4.480-109.790, p < 0.001), long disease duration (OR: 1.217, 95% CI: 1.024-1.447, p = 0.026), and high uric acid levels (OR: 1.868, 95% CI: 1.049-3.328, p = 0.034) were found to be related with the echocardiographic evidence of PH. Survival was worse in the PH group (p = 0.0001). CONCLUSION: Our results suggest that patients with myelofibrosis are more likely to develop PH than other MPNs patients. Disease duration may predict the development of PH in MPN patients.
BACKGROUND:Pulmonary hypertension (PH) can complicate the course of myeloproliferative neoplasms (MPNs). Echocardiography is a useful noninvasive screening test for PH in populations at risk. We aimed to investigate the echocardiographic evidence of PH and clinical characteristics of patients with MPNs. METHODS: This study included 197 patients with MPNs (mean age, 59 ± 14 years; females, 53%; mean disease duration, 3.4 ± 2.8 years). Clinical and laboratory characteristics, including JAK2V617F mutation status, were obtained. All participants underwent a comprehensive transthoracic echocardiographic examination. The echocardiographic evidence of PH was defined as systolic pulmonary artery pressure (SPAP) ≥40 mm Hg. RESULTS: Overall, 11 patients (5.5%) with SPAP ≥40 mm Hg had echocardiographic evidence of PH. Patients with myelofibrosis had echocardiographic evidence of PH more often than patients with other MPNs (p < 0.001). Disease duration since the diagnosis of MPNs was 6.7 ± 4.6 years in the PH group and 3.1 ± 2.5 years in the non-PH group (p < 0.001). There was a weak positive correlation between SPAP values and time since diagnosis (r = 0.236, p =0.001). JAK2V617F mutation was not associated with PH. In multivariate logistic regression analysis, the presence of myelofibrosis (odds ratio [OR]: 22.177, 95% CI: 4.480-109.790, p < 0.001), long disease duration (OR: 1.217, 95% CI: 1.024-1.447, p = 0.026), and high uric acid levels (OR: 1.868, 95% CI: 1.049-3.328, p = 0.034) were found to be related with the echocardiographic evidence of PH. Survival was worse in the PH group (p = 0.0001). CONCLUSION: Our results suggest that patients with myelofibrosis are more likely to develop PH than other MPNspatients. Disease duration may predict the development of PH in MPN patients.
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